Cystic Fibrosis and the Respiratory System

How does cystic fibrosis affect the respiratory system?

Cystic fibrosis (CF) is an inherited disease that causes problems in the cells of the body that make salt, water, and mucus. There is not a cure for CF. It is a disease that gets worse over time. But, improved treatments can help people with CF live longer and healthier. Most people with CF live into their late 30s, and many into their 50s. Some CF patients now live into their 70s. 

CF causes thick mucus to build up and clog certain parts of the body like the lung. The buildup is caused by an abnormal gene called the cystic fibrosis transmembrane regulator (CFTR). CFTR controls the flow of water and salt in and out of the body's cells. Changes cause mucus to become thickened and sticky. This first affects the small airways in the lungs. Eventually it can affect all airways.

Children with CF may develop lung infections because bacteria that are normally cleared from the lungs remain in the thickened mucus. Many of these lung infections are long-term (chronic). The most common bacteria that cause lung infections in people with CF is Pseudomonas aeruginosa.

Children with CF also can have problems in their upper respiratory tract. They can develop nasal polyps. These are small growths of tissue from the lining of the nose that extend into the nasal cavity. Sometimes these polyps must be taken out by a doctor. Children with CF also frequently have sinus infections.

A child with CF may have these symptoms, because of how CF affects the respiratory system: 

  • Chronic cough

  • Coughing up blood

  • Collapsed lung

  • Clubbing (The rounding and enlargement of the fingers and toes)

  • Heart enlargement

  • Nasal polyps

  • Sinusitis (Inflamed nasal sinuses)